Long Jun, Shi Yuchen, Yao Qiuyan, Zhou Xiaona, Luo Meihua, Shi Rongjie
The Clinical Medical College of Dali University, Dali, Yunnan Province, China.
First Affiliated Hospital of Kunming Medical University, Kunming, Yunnan Province, China.
Medicine (Baltimore). 2025 Jul 25;104(30):e43630. doi: 10.1097/MD.0000000000043630.
The prevalence of autoimmune liver disease in Sjogren syndrome (SS) ranges from 1.7% to 47%. Hematologic involvement is commonly observed in SS patients, with 1.5% of patients presenting with monoclonal gammopathy of undetermined significance (MGUS). Chronic antigenic stimulation of B lymphocytes in autoimmune diseases may eventually lead to clonal proliferation or MGUS. Nevertheless, studies reporting the concurrent manifestation of IgG4-related autoimmune hepatitis (IgG4-AIH), Primary biliary cholangitis (PBC), SS, and MGUS are scarce.
This article reports a rare case involving the overlap of multiple autoimmune diseases. A middle-aged female patient experienced fatigue, anorexia, xerostomia, jaundice, and pruritus, as well as elevated levels of liver enzymes, globulin, and monoclonal immunoglobulin (M protein).
Based on the findings of serum IgG4 levels, autoimmune serology, liver and labial gland biopsy, and bone marrow puncture, a diagnosis of IgG4-AIH, PBC, SS, and MGUS was made.
The patient was initiated on oral prednisone 40 mg once daily and ursodeoxycholic acid capsules 250 mg 3 times daily. Upon stabilization of the condition, prednisone was progressively tapered to a maintenance dose of 5 mg daily.
Over the 6-month follow-up period, favorable improvements were noted in the patient's symptoms.
The purpose of this report's research is to thoroughly describe the clinical manifestations, diagnostic challenges, and treatment difficulties associated with the rare co-existence of multiple autoimmune diseases and hematological diseases, and to summarize the management methods. The significance of this study lies in emphasizing the importance of screening for autoimmune diseases and hematological diseases in patients presenting similar symptoms in clinical practice, aiming to provide a reference for clinicians in identifying and managing such complex cases, thereby enhancing the understanding and treatment level of these diseases.
干燥综合征（SS）中自身免疫性肝病的患病率在1.7%至47%之间。血液系统受累在SS患者中很常见，1.5%的患者表现为意义未明的单克隆丙种球蛋白病（MGUS）。自身免疫性疾病中B淋巴细胞的慢性抗原刺激最终可能导致克隆性增殖或MGUS。然而，关于IgG4相关自身免疫性肝炎（IgG4-AIH）、原发性胆汁性胆管炎（PBC）、SS和MGUS同时出现的报道很少。
本文报告了一例罕见的涉及多种自身免疫性疾病重叠的病例。一名中年女性患者出现疲劳、厌食、口干、黄疸和瘙痒，以及肝酶、球蛋白和单克隆免疫球蛋白（M蛋白）水平升高。
根据血清IgG4水平、自身免疫血清学、肝脏和唇腺活检以及骨髓穿刺结果，诊断为IgG4-AIH、PBC、SS和MGUS。
患者开始口服泼尼松每日40毫克，熊去氧胆酸胶囊每日3次，每次250毫克。病情稳定后，泼尼松逐渐减量至每日5毫克的维持剂量。
在6个月的随访期内，患者症状有良好改善。
本报告的研究目的是全面描述与多种自身免疫性疾病和血液系统疾病罕见共存相关的临床表现、诊断挑战和治疗困难，并总结管理方法。本研究的意义在于强调在临床实践中对出现类似症状的患者进行自身免疫性疾病和血液系统疾病筛查的重要性，旨在为临床医生识别和管理此类复杂病例提供参考，从而提高对这些疾病的认识和治疗水平。
